Abstract
Definition and clinical pictureWe propose the minimal definition of Dercum’s disease to be generalised overweight or obesity in combination with painful adipose tissue. The associated symptoms in Dercum’s disease include fatty deposits, easy bruisability, sleep disturbances, impaired memory, depression, difficulty concentrating, anxiety, rapid heartbeat, shortness of breath, diabetes, bloating, constipation, fatigue, weakness and joint aches.ClassificationWe suggest that Dercum’s disease is classified into: I. Generalised diffuse form A form with diffusely widespread painful adipose tissue without clear lipomas, II. Generalised nodular form - a form with general pain in adipose tissue and intense pain in and around multiple lipomas, and III. Localised nodular form - a form with pain in and around multiple lipomas IV. Juxtaarticular form - a form with solitary deposits of excess fat for example at the medial aspect of the knee.EpidemiologyDercum’s disease most commonly appears between the ages of 35 and 50 years and is five to thirty times more common in women than in men. The prevalence of Dercum’s disease has not yet been exactly established.AetiologyProposed, but unconfirmed aetiologies include: nervous system dysfunction, mechanical pressure on nerves, adipose tissue dysfunction and trauma.Diagnosis and diagnostic methodsDiagnosis is based on clinical criteria and should be made by systematic physical examination and thorough exclusion of differential diagnoses. Advisably, the diagnosis should be made by a physician with a broad experience of patients with painful conditions and knowledge of family medicine, internal medicine or pain management. The diagnosis should only be made when the differential diagnoses have been excluded.Differential diagnosisDifferential diagnoses include: fibromyalgia, lipoedema, panniculitis, endocrine disorders, primary psychiatric disorders, multiple symmetric lipomatosis, familial multiple lipomatosis, and adipose tissue tumours.Genetic counsellingThe majority of the cases of Dercum’s disease occur sporadically. A to G mutation at position A8344 of mitochondrial DNA cannot be detected in patients with Dercum’s disease. HLA (human leukocyte antigen) typing has not revealed any correlation between typical antigens and the presence of the condition.Management and treatmentThe following treatments have lead to some pain reduction in patients with Dercum’s disease: Liposuction, analgesics, lidocaine, methotrexate and infliximab, interferon α-2b, corticosteroids, calcium-channel modulators and rapid cycling hypobaric pressure. As none of the treatments have led to long lasting complete pain reduction and revolutionary results, we propose that Dercum’s disease should be treated in multidisciplinary teams specialised in chronic pain.PrognosisThe pain in Dercum’s disease seems to be relatively constant over time.
Highlights
As none of the treatments have led to long lasting complete pain reduction and revolutionary results, we propose that Dercum’s disease should be treated in multidisciplinary teams specialised in chronic pain
Dercum’s disease is a rare disease listed by Orphanet [1] and by the National Organization for Rare Disorders (NORD) [2]
It is characterised by generalised overweight or obesity, pronounced pain in the adipose tissue and a number of associated symptoms
Summary
Dercum’s disease is a rare disease listed by Orphanet [1] and by the National Organization for Rare Disorders (NORD) [2]. Trentin et al [34] published a case of Dercum’s disease where the patient developed multiple painful adipose tissue lumps in the breasts. CT and MRI revealed diffuse thickening of the scalp tissue, but no evidence for other anomalies It is unclear what criteria were used when making the diagnosis Dercum’s disease and if the patient truly had Dercum’s disease. Kling has come up with the theory that adipose tissue deposits around the knees might interfere with the blood supply, by pressure on the joint, and result in the development of painful osteo-arthritis [43] It is unclear whether the juxta-articular form of Dercum’s disease exists, or if painful localised fat around the joints should be designated as the circumscribed diffuse form. Generalised nodular form: A form with general pain in adipose tissue and intense pain in and around multiple lipomas
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